History & exam

Key factors

  • facial plethora
  • supraclavicular fullness
  • violaceous striae
  • absence of pregnancy
  • menstrual irregularities
  • absence of malnutrition
  • absence of alcoholism
  • absence of physiologic stress
  • linear growth deceleration in children

Other diagnostic factors

  • female gender
  • hypertension
  • glucose intolerance or diabetes mellitus
  • premature osteoporosis or unexplained fractures
  • unexplained nephrolithiasis
  • weight gain and central obesity
  • acne
  • psychiatric symptoms
  • decreased libido
  • easy bruisability
  • weakness
  • facial rounding
  • dorsocervical fat pads
  • hirsutism

History & exam details

Diagnostic tests

1st tests to order

  • urine pregnancy test
  • serum glucose
  • late-night salivary cortisol
  • 1 mg overnight dexamethasone suppression test
  • 24-hour urinary free cortisol
  • 48-hour 2 mg (low-dose) dexamethasone suppression test

Tests to consider

  • plasma dehydroepiandrosterone sulfate (DHEAS) level
  • morning plasma ACTH
  • high-dose dexamethasone suppression test
  • pituitary MRI
  • adrenal CT
  • inferior petrosal sinus sampling
  • CT of chest, abdomen, and pelvis
  • MRI chest
  • PET scan
  • octreotide scanning

Diagnostic tests details

Treatment details

Ongoing

Cushing disease (ACTH-secreting pituitary tumor)

    • 1st line: transsphenoidal pituitary adenomectomy
    • adjunct: medical therapy before surgery
    • adjunct: postsurgical corticosteroid replacement therapy
    • adjunct: postsurgical non-corticosteroid pituitary hormone replacement therapy
    • 2nd line: repeat transsphenoidal pituitary adenomectomy
    • adjunct: medical therapy before surgery
    • adjunct: postsurgical corticosteroid replacement therapy
    • adjunct: postsurgical non-corticosteroid pituitary hormone replacement therapy
    • 3rd line: pituitary radiation therapy
    • adjunct: medical therapy before radiation therapy
    • adjunct: post-radiation corticosteroid replacement therapy
    • adjunct: post-radiation non-corticosteroid pituitary hormone replacement therapy
    • 4th line: bilateral adrenalectomy
    • plus: permanent postsurgical corticosteroid replacement therapy
    • adjunct: medical therapy before surgery
    • 4th line: medical therapy alone

ectopic ACTH or corticotropin-releasing hormone (CRH) syndrome

    • 1st line: surgical resection or ablation of tumor and metastases
    • adjunct: medical therapy
    • adjunct: chemotherapy or radiation therapy for primary tumor
    • 2nd line: bilateral adrenalectomy
    • plus: permanent postsurgical corticosteroid replacement therapy
    • adjunct: medical therapy before surgery
    • adjunct: chemotherapy or radiation therapy for primary tumor
    • 3rd line: medical therapy
    • adjunct: chemotherapy or radiation therapy for primary tumor

ACTH-independent due to unilateral adrenal carcinoma or adenoma

    • 1st line: unilateral adrenalectomy or tumor resection
    • adjunct: medical therapy before surgery
    • adjunct: chemotherapy or radiation therapy for adrenal carcinoma
    • 2nd line: medical therapy
    • adjunct: chemotherapy or radiation therapy for adrenal carcinoma

ACTH-independent due to bilateral adrenal disease (hyperplasia or adenoma)

    • 1st line: bilateral adrenalectomy
    • plus: permanent postsurgical corticosteroid replacement therapy
    • adjunct: medical therapy before surgery
    • 2nd line: medical therapy

Treatment details

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